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Five stories that will make you believe that, on their best days, doctors really do have superpowers.

August 2013

Paralyzed
Bacteria living in the most innocuous place almost end a young life.

 

Parents have gut feelings about their kids. They know when the house has suddenly become too quiet. They can hear a middle-of-the-night cry before it comes. And they’re able to tell when what looks like a run-of-the-mill cold is something much more sinister.
Kari and Brian Hinkel experienced what many people call a parent’s intuition this past January 1 when their five-month-old daughter, Keona, seemed crabby, was sleeping more than usual, and wasn’t eating well. To anyone else, these symptoms probably would’ve seemed less than alarming—and Kari admits she initially thought Keona had a head cold that was making her sluggish and making it difficult for her to breathe and breast-feed at the same time. She and Brian tried a few things: They put some saline solution in Keona’s nose, spoon-fed her some milk, and took a warm shower with her to see if the humidity would loosen her congestion. But less than 24 hours after her first symptoms, their daughter seemed to be getting worse. Kari knew something was wrong. The pretty, blue-eyed infant seemed like she wanted to eat, but couldn’t suck hard enough to breast-feed; she was becoming dehydrated; and she could barely keep her eyes open. They went to see the pediatrician, who told them to go to the ER.
At Sky Ridge Medical Center, ER docs asked the Hinkels patient history questions, took a look at Keona, and ran a few tests. They agreed that something wasn’t right, but they weren’t sure what it was. Then, without warning, Keona stopped breathing.

Sky Ridge physicians swiftly intubated the baby and then made the decision to fly her via helicopter to Rocky Mountain Hospital for Children (RMHC) at Presbyterian/St. Luke’s Medical Center in Uptown. In a state of shock, the Hinkels drove from Highlands Ranch to be with Keona in the ICU. Doctors at RMHC began a broad workup on the five-month-old, who was becoming weaker by the hour. Her eyes weren’t opening at all and she wasn’t moving. Doctors did an EEG and an MRI. They considered poisoning, seizures, and infections. But it wasn’t until one of the RMHC pediatric intensive care physicians, Dr. Jennifer Snow, consulted Dr. Benjamin Ross, a neurologist with special qualifications in child neurology, that a diagnosis began to take shape.
“I brought up infant botulism,” Ross says. “There’s a book of weird, rare diseases that it’s my job to know about—but I’ve actually seen infant botulism before. And this fit.” Common symptoms of this relatively uncommon paralytic illness—it’s seen about 90 times annually in the United States—are constipation, poor feeding, ptosis (eyelid droop), poor muscle tone, and lethargy. Infant botulism is caused when children younger than one consume spores of the botulinum bacterium, which are most often found in poorly oxygenated soil as well as in a household product nearly everyone has in the pantry: honey.
When children and adults get food-borne botulism, the result is usually a bout of unpleasant—but eminently survivable—food poisoning. But when an infant ingests the spores of Clostridium botulinum, the bacteria colonize a baby’s immature large intestine and produce a toxin that blocks the link between nerves and muscles. Essentially, the toxin creates a paralysis that begins at the head and moves its way down the body, sometimes causing babies to stop breathing. The fact that many physicians have never seen a case of infant botulism—which can be a contributing factor in a slow diagnosis—makes this a life-threatening situation.
In Keona’s case, the illness moved quickly—and she was fortunate her parents had followed their instincts. After all, there’s no better place to stop breathing than an emergency room. She was also lucky that Ross had seen infant botulism before. But that didn’t mean Keona would get better right away. Not only is the illness difficult to diagnose, it’s also challenging to confirm—and it’s even trickier to secure the medication needed to treat it.
For this part of the tag-team effort, Ross called upon RMHC infectious disease doctor Wendi Drummond. “It’s not a benign undertaking to diagnose and treat infant botulism,” Drummond says. “The treatment costs $45,000, there’s only one place we can get the antitoxin, and because there is a limited quantity of the medication, we have to be able to reasonably ‘prove’ we have the correct diagnosis before we can secure it.”

Lab results to verify infant botulism can take as long as a week. Clearly, that time frame was not ideal for a five-month-old on life support. She needed the antitoxin as soon as possible. Instead of waiting, Drummond set out to anecdotally confirm that Keona had somehow been exposed to botulism bacteria spores. “The patient history is critical in this situation,” Drummond explains. “I had to interview the baby’s mom.”
That, of course, is not an easy undertaking when a mother is as traumatized as 28-year-old Kari Hinkel had every right to be. But after asking Kari to think about what the family had done in previous weeks; what she had cooked for dinner; and whether she kept honey in the pantry, Drummond thought she had a plausible exposure scenario. The family had recently used honey to sweeten tea and Kari had also used it in a recipe just days before. It was possible Keona had somehow licked an errantly sticky finger.
Dr. Drummond hoped the information would be enough to acquire BabyBIG, the antitoxin, from the only place it was available: the California Department of Public Health Services’ Infant Botulism Treatment and Prevention Program. After a lot of paperwork and several in-depth phone calls—all of which took her nearly five hours to complete on the morning of January 4—Drummond had guaranteed that Keona would be getting the drug she needed. The medication arrived via commercial jet about seven hours later and was infused into Keona’s IV just two hours after that. Less than 48 hours after receiving BabyBIG, Keona began to move her legs and then her arms.
The Hinkels spent five weeks in the hospital. Keona was on a ventilator for 22 days before her body was strong enough to breathe on its own. The toxin that coursed through her system reverted her muscle tone back to that of a newborn. But after physical therapy, Keona began rolling over, sitting up, and, earlier this summer, crawling. She will celebrate her first birthday this month.

The Top Docs

Dr. Benjamin A. Ross
Child Neurology

Dr. Wendi Drummond
Pediatric Infectious Disease

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